Gluten-free diet and steroid treatment are effective therapy for most patients with collagenous sprue

Abstract

Background & aims: Collagenous sprue (CS) is characterized by the presence of a distinctive band of subepithelial collagen deposition in the small bowel. We evaluated the clinical characteristics, treatments, and outcomes of patients with CS.

Methods: Thirty patients with CS were identified at the 3 Mayo Clinic sites between 1993 and 2009. Clinical data from medical records were reviewed.

Results: The study cohort was 70% female (age range, 53-91 years). Most patients had severe diarrhea and weight loss. Hospitalization to treat dehydration was necessary in 16 (53%) patients. Associated immune-mediated diseases were noted in 70% of the patients; celiac disease was the most frequent. Other associated diseases were microscopic colitis, hypothyroidism, and autoimmune enteropathy. The median thickness of the layer of subepithelial collagen deposition in the small bowel was 29 mum (20-56.5 mum). Subepithelial collagen deposition in the colon or stomach was noted in 8 patients. A clinical response was observed in 24 (80%) patients after treatment with a combination of a gluten-free diet and immunosuppressive drugs. Histologic improvement was confirmed in 9 patients, with complete remission in 5. Two patients died (1 of complications of CS and 1 of another illness).

Conclusions: Most patients with CS are treated effectively with a combination of gluten-free diet and steroids. CS is often associated with collagen deposition or chronic inflammation in other segments of the gastrointestinal tract as well as other immune-mediated disorders.

Figure 1

Morphologic features of collagenous sprue. (A) Thickened subepithelial collagen layer (arrows). Note the absence of villi (total villous atrophy) and prominent surface epithelial injury (hematoxylin-eosin; original magnification, ×200). (B) Masson trichrome stain highlighted the thickened subepithelial collagen layer (arrows) (Masson trichrome; original magnification, ×200).

Figure 2

Complete remission of CS after treatment with a combination of GFD and budesonide. (A) Duodenal biopsy before treatment with thickened subepithelial collagen layer (arrows) and total villous atrophy (hematoxylin-eosin; original magnification, ×200). (B) Duodenal biopsy 2 years after treatment, with recovery of villi and absence of subepithelial collagen deposition (hematoxylin-eosin; original magnification, ×100).

Figure 3

Possible mechanisms of the pathogenesis of CS.